Table of Contents  
REVIEW ARTICLE
Year : 2014  |  Volume : 7  |  Issue : 2  |  Page : 88-95

Emergent management of Guillain-Barré syndrome


Department of Anesthesiology, Intensive Care, and Pain Management, Faculty of Medicine, Ain Shams University, Cairo, Egypt

Date of Submission18-Dec-2013
Date of Acceptance12-Jan-2014
Date of Web Publication31-May-2014

Correspondence Address:
Amr Mohamed EL-Said
Department of Anesthesiology, Intensive Care, and Pain Management, Faculty of Medicine, Ain Shams University, Abbassia, Cairo 11566
Egypt
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1687-7934.133302

Rights and Permissions
  Abstract 

Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy usually triggered by an infection. Ascending paralysis, weakness beginning in the feet and hands and migrating toward the trunk, is the most typical symptom, and some subtypes cause change in sensation or pain as well as dysfunction of the autonomic nervous system. This review aimed to revise knowledge and updates about this disease.

Keywords: Guillain-Barré syndrome, polyneuropathy, paralysis, neuro-critical care


How to cite this article:
EL-Said AM. Emergent management of Guillain-Barré syndrome. Ain-Shams J Anaesthesiol 2014;7:88-95

How to cite this URL:
EL-Said AM. Emergent management of Guillain-Barré syndrome. Ain-Shams J Anaesthesiol [serial online] 2014 [cited 2017 Dec 13];7:88-95. Available from: http://www.asja.eg.net/text.asp?2014/7/2/88/133302


  Introduction Top


Guillain-Barré syndrome (GBS), sometimes called Landry's paralysis or Guillain-Barré-Strohl syndrome, is an acute inflammatory demyelinating polyneuropathy, a disorder that affects the peripheral nervous system. Ascending paralysis, weakness beginning in the feet and hands and migrating toward the trunk, is the most typical symptom, and some subtypes cause change in sensation or pain as well as dysfunction of the autonomic nervous system. It can cause life-threatening complications, in particular if the breathing muscles are affected or if there is involvement of the autonomic nervous system. The disease is usually triggered by an infection [1].


  Classification Top


Six different subtypes of GBS exist [1]:

  1. Acute inflammatory demyelinating polyneuropathy is the most common form of GBS and the term is often used synonymously with GBS. It is caused by an autoimmune response directed against Schwann cell membranes.
  2. Miller-Fisher syndrome is a rare variant of GBS. Accounting for ∼5% of GBS cases, it manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS. It usually affects the eye muscles first and presents with the triad of ophthalmoplegia, ataxia, and areflexia. The ataxia predominantly affects the gait and trunk, with the limbs relatively spared. Anti-GQ1b antibodies are present in 90% of cases.
  3. Acute motor axonal neuropathy (AMAN) [2], also known as Chinese paralytic syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico. It is probably caused by an autoimmune response directed against the axoplasm of peripheral nerves, rather than the myelin sheath. The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies [3] are present. Anti-GD3 antibodies are found more frequently in AMAN.
  4. Acute motor sensory axonal neuropathy is similar to AMAN, but also affects sensory nerves, with severe axonal damage. Like AMAN, it is probably caused by an autoimmune response directed against the axoplasm of peripheral nerves. Recovery is slow and often incomplete [4].
  5. Acute panautonomic neuropathy is the most rare variant of GBS, sometimes accompanied by encephalopathy. It is associated with a high mortality rate, owing to cardiovascular involvement and associated dysrhythmias. Frequently occurring symptoms include impaired sweating, lack of tear formation, photophobia, dryness of nasal and oral mucosa, itching and peeling of skin, nausea, dysphagia, and constipation unrelieved by laxatives or alternating with diarrhea. Initial nonspecific symptoms of lethargy, fatigue, headache, and decreased initiative are followed by autonomic symptoms including orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhea, dryness of eyes, and disturbed micturition. The most common symptoms at onset are related to orthostatic intolerance as well as gastrointestinal (GI) and sudomotor dysfunction. Parasympathetic impairment (abdominal pain, vomiting, constipation, ileus, urinary retention, dilated unreactive pupils, loss of accommodation) may also be observed.
  6. Bickerstaff's brainstem encephalitis (BBE) is a further variant of GBS. It is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia, or Babinski's sign. The course of the disease can be monophasic or remitting-relapsing. Large, irregular hyperintense lesions located mainly in the brainstem, especially in the pons, midbrain, and medulla, are described in the literature. BBE, despite the severe initial presentation, usually has a good prognosis. MRI plays a critical role in the diagnosis of BBE. A considerable number of BBE patients have associated axonal GBS, indicative that the two disorders are closely related and form a continuous spectrum.



  Epidemiology Top


Worldwide, the annual incidence is about 0.6-4 occurrences per 100 000 individuals. Men are one and a half times more likely to be affected than women. The incidence increases with age; there is approximately one case per 100 000 individuals younger than 30 years of age and about four cases per 100 000 in those older than 75 years [5]. The incidence of GBS during pregnancy is 1.7 cases per 100 000 of the population [6]. Congenital and neonatal GBS have also been reported [7].


  History Top


The French physician Jean Landry first described the disorder in 1859. In 1916, Georges Guillain, Jean Alexandre Barré, and André Strohl diagnosed two soldiers with the illness and described the key diagnostic abnormality of increased spinal fluid protein production, but a normal cell count [1]. GBS is also known as acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French polio, Landry's ascending paralysis, and Landry Guillain-Barré syndrome. Canadian neurologist C. Miller-Fisher described the variant that bears his name in 1956 [1].


  Causes Top


All forms of GBS are because of an immune response to foreign antigens (such as infectious agents) that is mistargeted at host nerve tissues; instead, a phenomenon called molecular mimicry occurs. The targets of such immune attack are considered to be gangliosides, compounds naturally present in large quantities in human peripheral nerve tissues. The most common antecedent infection is the bacterium Campylobacter jejuni [8], followed by cytomegalovirus [9]. [Table 1] However, 60% of cases do not have a known cause. Some cases may be triggered by the influenza virus or by an immune reaction to the influenza virus [11]. There was an increased incidence of GBS following influenza immunization during the 1976-1977 swine flu pandemic [12]; however, epidemiological studies since then have shown either a very small increased risk following immunization (under one additional case per million vaccinations) or no increased risk [13].
Table 1: Commonly implicated antecedent infective agents in GBS [10]

Click here to view


The end result of this autoimmune attack on the peripheral nerves is damage to the myelin, the fatty insulating layer of the nerve, and a nerve conduction block, leading to muscle paralysis that may be accompanied by sensory or autonomic disturbances.

In mild cases, nerve axon (the long slender conducting portion of a nerve) function remains intact and recovery can be rapid if remyelination occurs. In severe cases, axonal damage occurs and recovery depends on the regeneration of this important tissue. Approximately 80% of patients have myelin loss; in the remaining 20%, the pathological hallmark is axon loss.

GBS, unlike disorders such as multiple sclerosis and Lou Gehrig's disease, is a peripheral nerve disorder and does not in general cause nerve damage to the brain or the spinal cord [1].

[TAG:2]Notable cases [1][/TAG:2]

fx1

  1. American actor Andy Griffith developed GBS in 1983. Griffith is seen here receiving an award at the White House in 2005.
  2. Markus Babbel, former international footballer, contracted GBS in 2001, following a period of suffering from the Epstein-Barr virus. He lost almost an entire year of his footballing career between the two illnesses and never demonstrated the same level of ability again that won him over 50 caps for Germany.
  3. Rachel Chagall, actress, contracted GBS in 1982. In 1987, she portrayed Gabriela Brimmer, a notable disabilities activist.
  4. Samuel Goldstein, American athlete and Paralympian.
  5. Joseph Heller, author, contracted GBS in 1981. This episode in his life is recounted in the autobiographical No Laughing Matter, which contains alternating chapters by Heller and his good friend Speed Vogel.
  6. Luci Baines Johnson, daughter of President Lyndon Johnson and Lady Bird Johnson, was diagnosed with and under treatment for GBS in April 2010.
  7. Hugh McElhenny, Pro Football Hall of Fame inductee and former professional American football player with the San Francisco 49ers.
  8. Lucky Oceans, Grammy Award-winning musician with Asleep at the Wheel, was diagnosed with GBS in 2008.
  9. Len Pasquarelli, sports writer and analyst for ESPN and resident of the Pro Football Writers of America, was diagnosed in 2008.
  10. Serge Payer, Canadian-born professional hockey player. After battling and overcoming the syndrome, he set up the Serge Payer Foundation, which is dedicated to raising money for research into new treatments and cures for GBS.
  11. William 'The Refrigerator' Perry, former professional American football player with the Chicago Bears, Super Bowl XX champion, and former Clemson University consensus first team All-American, was diagnosed with GBS in 2008.
  12. Franklin D. Roosevelt, US president. In 2003, a peer-reviewed study found that Roosevelt's paralysis - long attributed to poliomyelitis - was more likely caused by GBS.



  Signs and symptoms Top


The prodrome typically occurs between 0 and 8 weeks, with a peak interval of around 2-4 weeks. The disorder is characterized by symmetrical weakness that usually affects the lower limbs first and rapidly progresses in an ascending manner. Patients generally notice weakness in their legs, manifesting as 'rubbery legs' or legs that tend to buckle, with or without dysesthesias (numbness or tingling). As the weakness progresses upward, usually over periods of hours to days, the arms and facial muscles also become affected. Cranial nerve deficits are apparent in 45% of cases, with VII, IX, and X being the most frequently affected, leading to bulbar weakness, oropharyngealdysphagia (drooling or difficulty swallowing and/or maintaining an open airway), and respiratory difficulties. Most patients require hospitalization and about 30% require ventilatory assistance for the treatment of type II respiratory failure [14]. Facial weakness is also common. Eye movement abnormalities are not commonly seen in ascending GBS, but are a prominent feature in the Miller-Fisher variant.

Sensory loss, if present, usually takes the form of loss of proprioception (position sense) and areflexia (complete loss of deep tendon reflexes), an important feature of GBS. Loss of pain and temperature sensation is usually mild. In fact, pain is a common symptom in GBS, presenting as deep aching pain, usually in the weakened muscles, which patients compare with the pain from overexercising. These pains are self-limited and may be treated with standard analgesics. Bladder dysfunction may occur in severe cases, but is usually transient.

In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations in blood pressure, orthostatic hypotension (a decrease in blood pressure on standing, leading to an increased risk of collapse), and cardiac arrhythmias.

Acute paralysis in GBS may be related to sodium channel blocking factor in the cerebrospinal fluid (CSF). Significant issues involving intravenous salt and water administration may occur unpredictably in this patient group, resulting in syndrome of inappropriate anti-diuretic hormone hyper-secretion (SIADH), a cause of low sodium levels in the blood [1].

[TAG:2]Diagnosis [1][/TAG:2]

The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. CSF analysis (through a lumbar spinal puncture) and electrodiagnostic tests of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS.

Cerebrospinal fluid

Typical CSF findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated protein level (100-1000 mg/dl), without an accompanying increased cell count pleocytosis. Overall, 90% of patients will have a CSF protein level greater than 0.4 g/l after 1 week. A sustained increased white blood cell count may indicate an alternative diagnosis such as infection.

Electrodiagnostics

Electromyography and nerve conduction study may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.

[TAG:2]Diagnostic criteria [1][/TAG:2]

Required

  1. Progressive, relatively symmetrical weakness of two or more limbs because of neuropathy.
  2. Areflexia.
  3. Disorder course less than 4 weeks.
  4. Exclusion of other causes.


Supportive

  1. Relatively symmetric weakness accompanied by numbness and/or tingling.
  2. Mild sensory involvement.
  3. Facial nerve or other cranial nerve involvement.
  4. Absence of fever.
  5. Typical CSF findings obtained from lumbar puncture.
  6. Electrophysiologic evidence of demyelination from electromyogram.



  Prognosis Top


Most patients with GBS (74%) showed continued progression for up to 2 weeks, followed by a plateau phase of 2-4 weeks, and then recovery of function. Recovery usually starts after the fourth week from the onset of the disorder. At 6 months, about 65% of patients with GBS are able to walk independently. Overall, about 80% of patients with GBS either recover completely within a few months to a year or are left with only minor deficits that do not interfere with activities of daily living (ADLs) [15]. These minor deficits are observed in about 15% of patients and can vary from a mild foot drop or balance problems to moderate weakness or painful dysesthesias in the limbs.

About 5-10% recover with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage with inability of axonal regeneration [1].

Despite all improvements in treatment and supportive care, the death rate is still about 2-4% even in the best intensive care units, mostly from a lack of availability of life support equipment during the lengthy plateau lasting 4-6 weeks, and in some cases up to 1 year, when a ventilator is needed in the worst cases [1]. A 2008 epidemiological study reported 2-12% mortality despite ICU management [16]. Of patients who become ventilator dependent, about 20% will die. The causes of death include acute respiratory distress syndrome, sepsis, pulmonary emboli, and unexplained cardiac arrest [17].

About 5-10% of patients have one or more late relapses, with increased weakness, in which case, they are then classified as having chronic inflammatory demyelinating polyneuropathy [1].

Poor prognostic factors include the following [18]:

  1. Age over 40 years.
  2. History of preceding diarrheal illness.
  3. Weakness that is rapidly progressive over the first week.
  4. Respiratory failure requiring ventilator support.
  5. Average distal motor response amplitude reduction to less than 20% of normal.
  6. High anti-GM1 titer.
  7. Poor upper limb muscle strength.


Although initial studies suggested a possible correlation between anti-GM1 antibodies and poor recovery, a prospective trial found no such correlation [19].

Genetic or immunological host factors may play an important role in recurrent GBS as these patients can develop similar symptoms after different preceding infections. Recurrences occur more frequently in patients younger than 30 years of age, with milder symptoms.

Electrophysiology

Electrophysiology studies can have prognostic value, especially when repeated over the first 5 weeks. Axonal degeneration and poor prognosis (i.e. slower recovery and/or severe residual disability) are suggested by markedly reduced distal motor response amplitude (<20% of normal) and profuse fibrillation potentials on needle examination, starting at 2-4 weeks after disease onset. In contrast, demyelination and a good prognosis are associated with a pattern characterized by preservation of the distal motor response amplitude above 20% of normal, conduction block, and temporal dispersion [20].


  Principles of ICU management of GBS Top


The ICU management of GBS broadly consists of:

  1. Specific therapeutic interventions.
  2. General supportive measures in the interim of disease resolution.
  3. Avoidance of complications and iatrogenic insult.


General ICU supportive measures

Supportive care

Supportive care is very important in GBS as up to 30% of patients develop neuromuscular respiratory failure requiring mechanical ventilation. In addition, autonomic dysfunction may be severe enough to require ICU monitoring. Thus, many patients with GBS are initially admitted to the ICU for close monitoring of respiratory, cardiac, and hemodynamic function. Less severely affected patients can be managed in intermediate care units and mildly affected patients can be managed on the general ward with telemetry, along with monitoring of blood pressure and vital capacity every 4 h.

Prophylaxis for deep vein thrombosis, bladder and bowel care, physical and occupational therapy, and psychological support are essential. Subcutaneous fractionated or unfractionated heparin and support stockings are recommended until patients are able to walk independently. Adequate pain control is necessary [21].

Respiratory failure

Vigilance is essential when caring for a patient with GBS as deterioration because of progression of muscle weakness can occur rapidly.

Respiratory failure in GBS is common, and 15-30% of patients need ventilatory support. Thus, close respiratory monitoring with frequent measurement of vital capacity and negative inspiratory force should be instituted initially in all patients [21]. Bulbar dysfunction with swallowing problems and inability to clear secretions may add to the need for ventilatory support. The following parameters warn of impending respiratory failure and are an indication for intubation [22]:

  1. Forced vital capacity less than 15-20 ml/kg.
  2. Maximum inspiratory pressure less than 30 cmH 2 O.
  3. Maximum expiratory pressure less than 40 cmH 2 O.
  4. More than a 30% decrease in either forced vital capacity or maximum inspiratory pressure within 24 h.
  5. Time of onset to admission less than 7 days.
  6. Inability to cough.
  7. Inability to stand.
  8. Inability to lift the elbows.
  9. Inability to lift the head.
  10. Increases in liver enzyme.


In patients with at least four of these six predictors, mechanical ventilation was required in greater than 85%.

Weaning from mechanical ventilation should be guided by improvement in strength and serial pulmonary function tests (PFTs). Tracheostomy should be performed after 2 weeks if PFTs do not show any significant improvement from baseline, but can be deferred for another week if PFTs do show improvement [21]. Many intensivists will consider performing a tracheostomy at an early stage. One recognized advantage of an 'early' tracheostomy is a significant reduction in the amount of sedation that is required.

Autonomic dysfunction

Autonomic dysfunction is a well-recognized feature of GBS and is a significant source of mortality [23]. Dysautonomia occurs in 70% of patients and manifests as symptoms that include tachycardia (the most common), urinary retention, hypertension alternating with hypotension, orthostatic hypotension, bradycardia, other arrhythmias, ileus, and loss of sweating. Severe autonomic disturbances occur in about 20% of patients, mostly (but not always) in patients who develop severe weakness and respiratory failure. Consequently, close monitoring of blood pressure, fluid status, and cardiac rhythm is essential to the management of patients with GBS.

Cardiovascular management

In agreement with a 2005 expert review and consensus opinion of supportive care for patients with GBS, pulse and blood pressure monitoring has been recommended for patients with GBS who are becoming severely affected [21]. Monitoring should be continued until ventilatory support is no longer necessary or until recovery is underway in patients not needing mechanical ventilation. Practical tips for the management of patients with GBS include the following [24]:

  1. Quadriplegic patients should not be left unattended in the sitting position without assessment of orthostatic hypotension.
  2. Intravascular volume should be maintained, particularly during positive-pressure ventilation.
  3. Drugs with hypotensive side effects should be avoided if possible.
  4. Arrhythmias frequently occur during suctioning.
  5. Succinylcholine should be avoided.
  6. Plasma exchange can cause hypotension and electrolyte disturbances.


Blood pressure

Both paroxysmal hypertension and orthostatic hypotension are frequent, occurring in 24 and 19% of patients with GBS, respectively, whereas sustained hypertension occurs in 3% [24]. Intra-arterial monitoring should be instituted in the presence of significant blood pressure fluctuations. Hypotension can usually be treated with fluids and supine positioning, but low-dose phenylephrine can be used if fluids are not effective. In the presence of dysautonomia, only low doses of carefully titrated short-acting vasoactive agents should be used for the treatment of hypotension because of the potential to overshoot the target blood pressure in the setting of possible denervation hypersensitivity and these agents may produce markedly atypical responses in heart rate and blood pressure. Episodes of severe hypertension mean arterial pressure (MAP>125) can be treated with a short-acting β-blocker (labetalol, esmolol) or nitroprusside [24]. In cases of autonomic cardiovascular dysfunction, other conditions must be excluded, such as pulmonary thromboembolism, hypoxemia, sepsis, GI bleeding, and fluid and electrolyte disturbances [25].

Arrhythmias

Sustained sinus tachycardia occurs in 37% of patients and requires no treatment [24]. Severe cardiac arrhythmias, including bradycardia and asystole, occur in about 4% of patients with GBS. Temporary pacing may be required for patients with second-degree and third-degree heart block. Other arrhythmias and ECG changes have also been described: atrial fibrillation, atrial flutter, paroxysmal tachycardia, ventricular tachycardia, elevated or depressed ST segments, flat or inverted T waves, Q-T interval prolongation, axis deviation, and various conduction blocks [25]. Other causes of cardiovascular disease need to be excluded.

Bowel and bladder care

Additional autonomic problems include adynamic ileus and urinary retention. Daily abdominal auscultation to monitor for bowel silence and the development of adynamic ileus is recommended, as is monitoring of opioid administration. For the treatment of ileus, erythromycin or neostigmine may be effective [21]. Promotility agents are contraindicated in patients with dysautonomia.

Pain control

Neuropathic pain occurs in about 40-50 of patients during the course of GBS and often requires treatment. Pain may be relieved by frequent passive limb movements, gentle massage, and frequent position changes. Gabapentin or carbamazepine may be used for ICU pain control during the acute phase of GBS [21]. Simple analgesics or NSAIDS may be tried, but they often do not provide adequate pain relief. Appropriate narcotic analgesics may be used, but require careful monitoring for adverse effects (e.g. ileus) in the setting of autonomic denervation. Epidural morphine can also be useful [24]. For the long-term management of neuropathic pain, tricyclic antidepressants, tramadol, gabapentin, carbamazepine, mexiletine, or pregabalin may be useful [21].

Prevention of thromboembolism

Venous thromboembolism is one of the major sequelae of extremity paralysis. Time to development of deep vein thrombosis or pulmonary embolism varies from 4 to 67 days following the onset of symptoms [21]. Prophylaxis with gradient compression hose and subcutaneous heparin or low-molecular-weight heparin may markedly reduce the incidence of venous thromboembolism [21].

True gradient compression stockings (30-40 mmHg or higher) are highly elastic and provide compression along a gradient that is highest at the toes and gradually decreases to the level of the thigh. This reduces capacity venous volume by ∼70% and increases the measured velocity of blood flow in the deep veins by a factor of 5 or more. The ubiquitous white stockings known as antiembolic stockings or thromboembolic disease hose produce a maximum compression of 18 mmHg and rarely are fitted in such a way as to provide adequate gradient compression. They have not been shown to be effective as prophylaxis against thromboembolism.

Nutrition

GI support should include nasogastric feeding wherever possible. Problems may occur when an ileus is present, and the need for airway protection should again be considered when bulbar palsy and gastric reflux is a possibility. Total parenteral nutrition may occasionally be necessary when enteral measures prove inadequate [10].

Rehabilitation

Acute-phase rehabilitation should include an individualized program of gentle strengthening, involving isometric, isotonic, isokinetic, and manual resistive and progressive resistive exercises [21]. Rehabilitation should emphasize proper limb positioning, posture, and orthotics. A device to help with communication may be necessary.

GBS patients frequently require a great deal of psychological support, given the extremely disabling and frightening aspects of the condition and its sequelae. Undertaking a multidisciplinary approach to GBS patients is an important aspect of overall ICU management, but is particularly useful in addressing patients' psychological well-being [10].

Following the acute phase, treatment often consists of rehabilitation with the help of a multidisciplinary team to focus on improving ADLs. Occupational therapists may offer equipment (such as wheelchair and special cutlery) to help the patient achieve ADL independence. Physiotherapists assist to correct functional movement, avoiding harmful compensations that might have a negative effect in the long run. There is also some evidence supporting physiotherapy in helping patients with GBS regain strength, endurance, and gait quality, as well as helping them prevent contractures, bedsores, and cardiopulmonary difficulties [26]. Speech and language therapists help regain speaking and swallowing ability, especially if the patient was intubated or received a tracheostomy.

Specific therapeutic interventions

Disease modifying treatment

Two specific treatment approaches are used to combat GBS:

(1) Plasmapheresis: this process involves removing blood from the body, separating the cellular elements of the blood, resuspending these cellular elements in a plasma substitute, and then reinfusing this mixture into the body. The purpose of this procedure is to 'cleanse' the blood of antibodies, complement, and soluble biological response modifiers directed against the myelin sheaths of peripheral nerves [27],[28].

(2) Intravenous immune globulin (IVIG): this treatment involves the administration of high doses of intravenous immunoglobulin 0.4 g/kg. The precise mechanism of action for IVIG in GBS is unknown, but may include providing anti-idiotypic antibodies, modulating the expression and function of Fc receptors, interfering with the activation of complement and production of cytokines, and interfering with activation and effector functions of T and B cells [29].

Plasma exchange

Large, randomized multicenter trials have established the effectiveness of plasma exchange in patients with severe GBS [27],[28]. Earlier improvement in muscle strength reduced the need for mechanical ventilation, and better recovery has been shown. Plasma exchange is usually administered for four to six treatments over 8-10 days, for a total of 200-250 ml/kg. The main complications are hypotension, sepsis, and problems with intravenous access [30].

  1. Plasma exchange was most effective when started within 7 days of symptom onset. However, in the North American study that allowed enrollment up to 30 days after the onset of symptoms, there was still an improvement in outcome in the plasma exchange group compared with the controls.
  2. Two plasma exchanges were superior to none in patients with mild GBS, and four exchanges were superior to two in patients with moderately severe GBS. However, in patients with severe disease requiring mechanical ventilation, six exchanges were not superior compared with four.


Intravenous immune globulin

IVIG is as effective as plasma exchange for the treatment of GBS when started within 2 weeks of the onset of symptoms [30],[31]. IVIG is usually used first because of its ease of administration and safety profile. IVIG is administered for 5 days at 0.4 g/kg/day. Patients with more severe clinical disease may benefit from a longer duration (6 days) of IVIG treatment [32]. The combination of IVIG with plasma exchange is not significantly better than either alone [33]. Side effects include hepatitis, aseptic meningitis, rash, acute renal failure (mostly related to sucrose-containing products), and (rarely) hyperviscosity leading to stroke. Immunoglobulin A deficiency can lead to anaphylaxis [29].

Other therapies

Glucocorticoids

Once the mainstay of therapy for GBS, glucocorticoids have not been shown to be beneficial and no longer play a role [34]. Also, combined treatment with intravenous methylprednisolone and IVIG showed no significant benefit compared with IVIG alone for patients with GBS [35].

Investigational agents

In an animal model, intravenous eculizumab was reported to prevent respiratory paralysis and the functional and morphological hallmarks of terminal motor neuropathy. Human studies have not been reported [36].

Interferon-β was not associated with a significant clinical improvement compared with controls in a small randomized-controlled trial [37]. Mycophenolatemofetil, used as an adjunct therapy with IVIG, showed no benefit in a pilot study [38].

Immune adsorption

Immune adsorption is an alternative treatment for GBS that is still in the early stages of investigation. A small prospective study reported no difference in outcome between patients treated with immunoadsorption and those treated with plasma exchange [39].

In critically ill patients, a small German study reported that treatment with selective immune adsorption seems to be safe and effective. In comparison with treatment with selective immune adsorption only, sequential therapy with IVIG was not more effective [40].

Guideline recommendations

The American Academy of Neurology practice parameter on immunotherapy for GBS published in 2003 recommended the following [30]:

  1. Patients recover sooner and better when treated early.
  2. Plasma exchange is recommended for nonambulatory adult patients with GBS who start treatment within 4 weeks of the onset of neuropathic symptoms. Plasma exchange is also recommended for ambulatory patients who start treatment within 2 weeks of the onset of neuropathic symptoms.
  3. IVIG is recommended for nonambulatory adult patients with GBS who start treatment within 2 or possibly 4 weeks of the onset of neuropathic symptoms.
  4. The choice between plasma exchange and IVIG is dependent on local availability and on patient-related risk factors, contraindications, and preference. Because of its ease of administration and wide availability, IVIG is frequently the preferred treatment.


Immunizations

Although GBS has followed vaccinations, the actual risk of vaccine-associated GBS is uncertain. In general, for vaccination in patients with GBS, the following recommendations should be considered [21]:

  1. Immunizations are not recommended during the acute phase of GBS and are not suggested for a period of 1 year or more after the onset of GBS.
  2. After 1 year, immunizations need not be withheld, but the need for the immunization should be reviewed on an individual basis; the established benefits of vaccination justify yearly vaccination.
  3. Future avoidance is suggested for any particular immunization that is followed within 6 weeks by the onset of GBS.



  Acknowledgements Top


Conflicts of interest

None declared.

 
  References Top

1.Davids H. Guillain-Barré syndrome. ??? 2012; ???:???-???.  Back to cited text no. 1
    
2. McKhann GM, Cornblath DR, Ho T, et al. Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China. Lancet 1991; 338:593-597.  Back to cited text no. 2
    
3. Ho TW, Mishu B, Li CY, et al. Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain 1995; 118:597-605.  Back to cited text no. 3
    
4. Griffin JW, Li CY, Ho TW, et al. Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. Brain 1995; 118:577-595.  Back to cited text no. 4
    
5. Pithadia AB, Kakadia N. Guillain-Barré syndrome (GBS). Pharmacol Rep 2010; 62:220-232.  Back to cited text no. 5
    
6. Brooks H, Christian AS, May AE. Pregnancy, anaesthesia and Guillain-Barré syndrome. Anaesthesia 2000; 55:894-898.  Back to cited text no. 6
    
7. Iannello S. Guillain-Barré syndrome: pathological, clinical and therapeutical aspects 2004; Nova Publishers ???.  Back to cited text no. 7
    
8. Yuki N. Campylobacter genes responsible for the development and determinant of clinical features of Guillain-Barré syndrome. Nippon Rinsho 2008; 66:1205-1210.  Back to cited text no. 8
[PUBMED]    
9. Orlikowski D. Guillain-Barré syndrome following primary cytomegalovirus infection: a prospective cohort study. Clin Infect Dis 2011; 52:837-844.  Back to cited text no. 9
    
10.1Craw N. Guillain-Barré syndrome (GBS). Anaesthesia 2008; ???:???-???.  Back to cited text no. 10
    
11.1Sivadon-Tardy V, et al. Guillain-Barré syndrome and influenza virus infection. Clin Infect Dis 2009; 48:48-56.  Back to cited text no. 11
    
12.1Haber P, et al. Guillain-Barré syndrome following influenza vaccination. JAMA 2004; 292:2478-2481.  Back to cited text no. 12
    
13.1Liang XF, et al. Safety of influenza A (H1N1) vaccine in postmarketing surveillance in China. N Engl J Med 2011; 364:638-647.  Back to cited text no. 13
    
14.1Burt CC, Arrowsmith JE. Respiratory failure. Surgery 2009; 27:475-479.  Back to cited text no. 14
    
15.1Ropper AH, Wijdicks EFM, Truax BT. Guillain-Barré syndrome. Philadelphia: FA Davis; 1991.  Back to cited text no. 15
    
16.1Alshekhlee A, Hussain Z, Sultan B, et al. Guillain-Barré syndrome: incidence and mortality rates in US hospitals. Neurology 2008; 70:1608-1613.  Back to cited text no. 16
    
17.1Lawn ND, Wijdicks EF. Fatal Guillain-Barré syndrome. Neurology 1999; 52:635-638.  Back to cited text no. 17
    
18.1Van Koningsveld R, Steyerberg EW, Hughes RA, et al. A clinical prognostic scoring system for Guillain-Barré syndrome. Lancet Neurol 2007; 6:589-594.  Back to cited text no. 18
    
19.1Rees JH, Gregson NA, Hughes RA. Anti-ganglioside GM1 antibodies in Guillain-Barré syndrome and their relationship to Campylobacter jejuni infection. Ann Neurol 1995; 38:809-816.  Back to cited text no. 19
    
20.2Albers JW. AAEE case report #4: Guillain-Barré syndrome. Muscle Nerve 1989; 12:705-711.  Back to cited text no. 20
    
21.2Hughes RA, Wijdicks EF, Benson E, et al. Supportive care for patients with Guillain-Barré syndrome. Arch Neurol 2005; 62:1194-1198.  Back to cited text no. 21
    
22.2Lawn ND, Fletcher DD, Henderson RD, et al. Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol 2001; 58:893-898.  Back to cited text no. 22
    
23.2Hund EF, Borel CO, Cornblath DR, et al. Intensive management and treatment of severe Guillain-Barré syndrome. Crit Care Med 1993; 21:433-446.  Back to cited text no. 23
    
24.2Ropper AH. In: Ropper AH, ed. Critical care of Guillain-Barré syndrome. Neurological and neurosurgical intensive care. 4 th ed.. Philadelphia: Lippincott Williams & Wilkins; 2003; 278-298.  Back to cited text no. 24
    
25.2Zochodne DW. Autonomic involvement in Guillain-Barré syndrome: A review. Muscle Nerve 1994; 17:1145-1155.  Back to cited text no. 25
    
26.2Davidson I, Wilson C, Walton T, Brissenden S. Physiotherapy and Guillain-Barré syndrome: results of a national survey. Physiotherapy 2009; 95:157-163.  Back to cited text no. 26
    
27.2Raphael JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev 2002; ???:???-???.  Back to cited text no. 27
    
28.2Hughes RA, Swan AV, Raphael JC, et al. Immunotherapy for Guillain-Barré syndrome: a systematic review. Brain 2007; 130:2245-2257.  Back to cited text no. 28
    
29.2Dalakas MC. The use of intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: evidence-based indications and safety profile. Pharmacol Ther 2004; 102:177-193.  Back to cited text no. 29
    
30.3Hughes RA, Wijdicks EF, Barohn R, et al. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2003; 61:736-740.  Back to cited text no. 30
    
31.3Hughes RA, Raphael JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database. Cochrane Database Syst Rev 2006; ???:???-???.  Back to cited text no. 31
    
32.3Raphael JC, Chevret S, Harboun M, Jars-Guincestre MC. Intravenous immune globulins in patients with Guillain-Barré syndrome and contraindications to plasma exchange: 3 days versus 6 days. J Neurol Neurosurg Psychiatry 2001; 71:235-238.  Back to cited text no. 32
    
33.3Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Lancet 1997; 349:225.  Back to cited text no. 33
    
34.3Hughes RA, Swan AV, van Koningsveld R, van Doorn PA. Corticosteroids for Guillain-Barré syndrome. Cochrane Database Syst Rev 2006; ???:???-???.  Back to cited text no. 34
    
35.3VanKoningsveld R, Schmitz PI, Meche FG, et al. Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial. Lancet 2004; 363:192-196.  Back to cited text no. 35
    
36.3Halstead SK, Zitman FM, Humphreys PD, et al. Eculizumab prevents anti-ganglioside antibody-mediated neuropathy in a murine model. Brain 2008; 131:1197-1208.  Back to cited text no. 36
    
37.3Pritchard J, Gray IA, Idrissova ZR, et al. A randomized controlled trial of recombinant interferon-beta 1a in Guillain-Barré syndrome. Neurology 2003; 61:1282-1284.  Back to cited text no. 37
    
38.3Garssen MP, van Koningsveld R, van Doorn PA, et al. Treatment of Guillain-Barré syndrome with mycophenolatemofetil: a pilot study. J Neurol Neurosurg Psychiatry 2007; 78:1012-1013.  Back to cited text no. 38
    
39.3Seta T, Nagayama H. Katsura K. Factors influencing outcome in Guillain-Barré Syndrome: comparison of plasma adsorption against other treatments. Clin Neurol Neurosurg 2005; 107:491-496.  Back to cited text no. 39
    
40.4Galldiks N, Dohmen C, Neveling M, et al. Selective immune adsorption treatment of severe Guillain-Barré syndrome in the intensive care unit. Neurocrit Care 2009; ???:???-???.  Back to cited text no. 40
    



 
 
    Tables

  [Table 1]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
   Abstract
  Introduction
  Classification
  Epidemiology
  History
  Causes
  Notable cases [1]
  Signs and symptoms
  Diagnosis [1]
   Diagnostic crite...
  Prognosis
   Principles of IC...
  Acknowledgements
   References
   Article Tables

 Article Access Statistics
    Viewed2494    
    Printed31    
    Emailed1    
    PDF Downloaded401    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]